(In)Distinctive Role of Long Non-Coding RNAs in Common and Rare Ovarian Cancers

SIMPLE SUMMARY: Ovarian cancers (OCs) are the most lethal form of gynecological tumors. The commonest are high-grade serous OCs, while rare OCs originate from many different cell types, such as epithelial, germ cell, sex cord-stromal, or mixed types. Rare OCs have distinct molecular characteristics, prognosis, and therapeutic approaches. However, all ovarian malignancies mostly share the same problem: late diagnosis due to the lack of specific symptoms. Therefore, there is a perpetual need to discover better diagnostic, prognostic, and predictive biomarkers, as well as new therapeutic approaches. In recent years, long non-coding RNAs (lncRNAs) have gained widespread attention because of their important role in various biological pathways. They have multiple mechanisms of action with an important role in many cellular processes related to OCs development and progression. This review will focus on the different aspects of lncRNAs in OCs and attempt to highlight the distinctive role of lncRNAs in common and rare OCs. ABSTRACT: Rare ovarian cancers (ROCs) are OCs with an annual incidence of fewer than 6 cases per 100,000 women. They affect women of all ages, but due to their low incidence and the potential clinical inexperience in management, there can be a delay in diagnosis, leading to a poor prognosis. The underlying causes for these tumors are varied, but generally, the tumors arise due to alterations in gene/protein expression in cellular processes that regulate normal proliferation and its checkpoints. Dysregulation of the cellular processes that lead to cancer includes gene mutations, epimutations, non-coding RNA (ncRNA) regulation, posttranscriptional and posttranslational modifications. Long non-coding RNA (lncRNA) are defined as transcribed RNA molecules, more than 200 nucleotides in length which are not translated into proteins. They regulate gene expression through several mechanisms and therefore add another level of complexity to the regulatory mechanisms affecting tumor development. Since few studies have been performed on ROCs, in this review we summarize the mechanisms of action of lncRNA in OC, with an emphasis on ROCs.